Fibrolamellar carcinoma (FLC) is a rare form of liver cancer which is generally diagnosed in adolescents and young adults (before age 40). Many people with early FLC have no signs or symptoms of the condition. When present, symptoms are often nonspecific (i.e. abdominal pain, weight loss, malaise) and blamed on other, more common conditions. The exact underlying cause of FLC is poorly understood. Unlike other forms of liver cancer, FLC typically occurs in the absence of underlying liver inflammation or scarring; thus, specific risk factors for this condition remain unidentified. FLC is typically treated with surgical resection.[1][2][3]

Many people with early fibrolamellar carcinoma (FLC) have no signs or symptoms of the condition. When present, symptoms are often nonspecific and blamed on other, more common conditions. Signs and symptoms may include:[1][2][4][5]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have
all the symptoms listed. This information comes from a database called the Human Phenotype Ontology
(HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO
is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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HPO ID

Percent of people who have these symptoms is not available through HPO

The exact underlying cause of fibrolamellar carcinoma (FLC) is poorly understood. Other forms of liver cancer are often associated with liver cirrhosis (scarring of the liver) which may be caused by alcohol abuse; autoimmune diseases of the liver; Hepatitis B or C viral infections; chronic inflammation of the liver; and/or hemochromatosis. However, FLC typically occurs in the absence of underlying liver inflammation or scarring; thus, specific risk factors for this condition remain unidentified.[1][2][3]

Recent research suggests that a deletion on chromosome 19 may play a key role in the formation of FLC.[6] This deletion is called a "somatic mutation" since it is only present in the cells of the liver. Somatic mutations accumulate during a person's lifetime and are not inherited or passed on to future generations.

If fibrolamellar carcinoma (FLC) is suspected based on the presence of certain signs and symptoms, imaging studies such as ultrasound, MRI scan and/or CT scan are typically recommended for diagnosis and staging. Unlike other forms of liver cancer, serum alpha fetoprotein is typically not elevated in FLC.[1][2]

Medscape Reference's Web site offers more specific information on the diagnosis of FLC. Please click on the link to access this resource.

The standard treatment for fibrolamellar carcinoma (FLC) is surgical resection. Due to the rarity of the condition, there is limited information to support the use of other treatment options and there is no standard chemotherapy regimen. However, other treatments may be considered if surgical resection isn't an option. For example, liver transplantation may be considered in patients who are not candidates for partial resection (removing a portion of the liver).[1][2]

Medscape Reference's Web site offers more specific information on the treatment and management of FLC. Please click the link to access this resource.

Last updated: 4/23/2015

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.
Learn more orphan products.

The long-term outlook (prognosis) for people with fibrolamellar carcinoma (FLC) is generally better than other forms of liver cancer, particularly when treated with surgical resection.[2][1] In fact, reported 5-year survival rates range from 51 to 70% following liver resection.[7][8][9][2]

Unfortunately, the prognosis is less favorable in people with unresectable disease. The median survival of these patients is approximately 14 months.[1]

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Fibrolamellar carcinoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

The Fibrolamellar Tissue Repository is a tissue bank for fibrolamellar samples obtained during surgery. This repository is a resource for Rockefeller University and other approved academic institutions who are interested in performing scientific research that will improve the diagnosis, treatment, and understanding of this rare disease.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Financial Resources

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.

The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.

Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.

PubMed is a searchable database of medical literature and lists journal articles that discuss Fibrolamellar carcinoma. Click on the link to view a sample search on this topic.

Other Conferences

The National Institute of Diabetes and Digestive and Kidney Diseases, National Cancer Institute, National Institute of Biomedical Imaging and Bioengineering, Department of Veteran Affairs, and Fogarty International Center sponsored a conference on hepatocellular carcinoma on April 1-3, 2004 to discuss the screening, diagnosis, and management of this type of cancer. You can view the agenda as well as a list of speakers by clicking here.

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